Failed Newborn Hearing Screening Research Articles

Impact of Cytomegalovirus (CMV) on an Academic Pediatric Infectious Diseases Outpatient Clinic Referral Population, 2005-2020: Will the Advent of Universal Congenital CMV (cCMV) Screening Change Clinical Practice Referral Patterns?

Cytomegalovirus (CMV) infections exert a substantial impact on the practice of pediatric infectious diseases. Although most infections in children are minimally symptomatic, several populations are at risk for CMV-associated disease, including immunosuppressed children, children with HIV infection, and, most significantly, children with congenital CMV (cCMV) infection. In spite of the ubiquitous nature of CMV infection, few studies have quantified the impact of CMV-associated care in a pediatric outpatient clinic setting. We evaluated the impact of CMV on clinical care in an outpatient clinic setting over a fifteen-year period at the University of Minnesota (UMN) Masonic Children's Hospital Pediatric Infectious Diseases (PID) Clinic. A retrospective review of clinic appointments identified 253 unique patients specifically evaluated over this time period for consideration of CMV infection. Of these, 242 were pediatric patients. The majority of the pediatric patients evaluated in the PID clinic were referred for either confirmed or suspected cCMV infection, including children referred for consideration of CMV as a potential reason for a failed newborn hearing screen (NHS) and/or for evaluation of CMV as a possible etiology for documented hearing loss. In total, 116 of the children evaluated during this time period (48%) were unequivocally confirmed as having cCMV infection, with an additional 37 (15%) presenting with presumed, probable, or possible cCMV infection. A total of 16 (7%) of the pediatric CMV cases were confirmed to be post-natally acquired infections. Of the 253 total patients, 11 (4%) of the referrals were for pregnant patients seeking advice about potential therapies in the setting of a known or suspected primary maternal infection during their pregnancies, with an attendant risk of fetal CMV infection. This overview of the demographics and referral patterns for patients evaluated for known or suspected CMV infections in a tertiary care center outpatient PID clinic will serve as a useful baseline assessment, even as future patterns of outpatient care are highly likely to evolve. We predict that PID clinic referrals for newborns identified by universal cCMV screening programs will result in a shift of the CMV outpatient population to healthier infants with clinically inapparent infections, and care will need to be taken by practitioners not to over-medicalize management for these asymptomatic newborns.

Hearing Outcomes in Failed Newborn Hearing Screening Infants With and Without Chronic Serous Otitis.

To identify outcomes in hearing loss (HL) diagnosis and intervention in infants with a failed newborn hearing screen (NBHS) and otitis media with effusion (OME) compared to those with failed NBHS and without OME. Retrospective review. Tertiary medical center. A chart review was performed on infants referred to Primary Children's Hospital for failed NBHS from 2012 to 2018. Eighty infants with failed NBHS and OME and 55 with failed NBHS and no OME were included. Incidence of permanent HL along with the age of HL confirmation and early intervention (EI) enrollment were compared. The incidence of OME in infants with failed NBHS was 59.3%. Fifty-six percent of infants with OME and 12.5% of those without OME did not receive definitive hearing confirmation in either ear due to loss to follow-up or insufficient audiometric assessment. Permanent HL was identified in 11.3% (n = 9) of infants with OME and in 20.0% (n = 11) of those without OME. Infants with OME were significantly older at the time of HL confirmation (4.2 ± 2.1 months) and EI enrollment (5.4 ± 2.5 months) compared to those without OME at the time of HL confirmation (1.0 ± 1.0 months; p < .001) and EI enrollment (2.6 ± 1.8 months; p = .04). Infants with failed NBHS and OME are highly susceptible to a significant delay in HL confirmation or lack of confirmatory hearing tests. Timely OME resolution with earlier ventilation tube insertion by 3 months of age and follow-up audiologic assessment is recommended.

How Audiologists Can Support EHDI Goals

How Audiologists Can Support EHDI Goals

Communities Helping the Hearing of Infants by Reaching Parents (CHHIRP) through patient navigation: a hybrid implementation effectiveness stepped wedge trial protocol

IntroductionAs the most common neonatal sensory disorder in the USA, infant hearing loss has an incidence of 1.7 per 1000 births. The consequences of delayed diagnosis and failure to obtain...

Early Hearing Detection and Intervention for Children with Craniofacial Anomalies

Early Hearing Detection and Intervention for Children with Craniofacial Anomalies

Expanding the Role of Educational Audiologists After a Failed Newborn Hearing Screening: A Quality Improvement Study.

Purpose Lack of timely and proximal access to diagnostic hearing evaluation using auditory brainstem response (ABR) testing hampers the effectiveness of Early Hearing Detection and Intervention (EHDI) programs in the United States. This study measured the impact of a state-based quality-improvement (QI) project that provided diagnostic ABR equipment and training to educational audiologists distributed throughout Iowa in regional special education centers. Method We used de-identified administrative data generated by the state EHDI program to analyze markers of access to early hearing health care for infants in a preproject condition ("Baseline") compared to the implementation of diagnostic ABRs at the regional special education centers ("QI Project"). Results Our findings revealed that the QI Project was associated with improvements in timeliness of first hearing evaluation, distance traveled for first hearing evaluation, and likelihood of receiving on-guideline audiology care during the first hearing evaluation. Conclusions Following the onset of the QI Project, infants and their families had greater access to initial hearing evaluation after failed newborn hearing screening. This improvement could have cascading effects on timeliness of later intervention among those with confirmed permanent childhood hearing loss.

Congenital and Neonatally Acquired Sensorineural Hearing Loss.

Hearing loss is one of the most common congenital diagnoses, recognized in large part by a robust newborn hearing screening program. Ensuring appropriate follow-up of failed newborn hearing screening is crucial to avoid delay in initiation of treatment for hearing loss. The most common etiology for congenital hearing loss is genetic, but some cases can be acquired. Understanding of the etiology aids in counseling for the family and in direction of treatment. Early diagnosis and treatment results in dramatically improved speech and developmental outcomes for affected children. Treatment including amplification, speech therapy, adaptations in the classroom, and family support leads to gains in academic performance, parental satisfaction, and quality of life. Early cochlear implantation has been shown to be beneficial in obtaining speech and language skills in patients with severe to profound sensorineural hearing loss and should be considered in the appropriate patient population. [Pediatr Ann. 2021;50(7):e292-e296.].

Association between Hyperbilirubinemia and Hearing Screen Failure in the Neonatal Intensive Care Unit in Infants Born Preterm

To characterize the association between hyperbilirubinemia and a failed newborn hearing screen in infants born at 22-32weeks of gestation. We included infants with gestational ages of 22-32weeks who were discharged from neonatal intensive care units in the US from 2002 to 2017 with available newborn hearing screen results obtained after 34weeks postmenstrual age. We excluded infants with severe birth asphyxia or craniofacial abnormalities. We identified 95 672 infants from 313 neonatal intensive care units. We used multivariable logistic regression to examine the association between maximum total bilirubin at <21days postnatal age with failed hearing screen, adjusting for important demographic and clinical risk factors. The median gestational age and birth weight were 30weeks (IQR, 28-32weeks) and 1330g (IQR, 1010-1630g), respectively. The median maximum total bilirubin was 8.3mg/dL (IQR, 6.7-10.0mg/dL), and 5275 infants (6%) failed their newborn hearing screen. On adjusted analysis, each 1mg/dL increase in maximum total bilirubin was associated with a small, but significant, increase in odds of a failed hearing screen (OR, 1.03; 95% CI, 1.02-1.04). An increased maximum total bilirubin level was independently associated with hearing screen failure. Further prospective studies are needed to understand whether this increased risk of hearing screen failure translates to increased risk of hearing loss.

P34 An audit of the testing and documentation of genetic mutation m1555a&gt;g and aminoglycoside prescribing

AimsAmikacin is an aminoglycoside antibacterial, associated with both ototoxicity and nephrotoxicity.1 It is used at the Trust first line for many indications, such as sepsis and pre-operative prophylaxis. A mutation in the m1555A&gt;G gene has been linked to an increased risk of aminoglycoside induced ototoxicity2, therefore making these patients susceptible to hearing damage when treated with aminoglycosides.3 The residual effect of this damage is both profound and rapidly progressive with resulting permanent hearing loss occurring even when the doses administered remain in the therapeutic window.4Trust guidelines containing amikacin advise that genetic testing should consistently be carried out and where this is not possible, clinicians should discuss and receive advice from the microbiology team for an alternative option. Genetic testing can take up to two weeks to be reported causing a logistical problem for patients requiring the medication usually on a more urgent basis.The aim of this study was to assess whether the Trust routinely provides genetic testing for mitochondrial DNA mutation m.1555A&gt;G abnormality and to determine whether prescribed antibiotics are appropriately following the results of this testing, including any resulting patient safety issues.MethodThe data was collected retrospectively from all genetic testing results since 2008 using prescribing data from the implementation of JAC and also an analysis of patient notes to check audiology referrals. The comparative standards used were 100% of positive test results should be recorded on patient prescribing record, this process is currently manual. 100% of patients who have received an aminoglycoside have prior testing for the m1555A&gt;G mutation, 100% of patients who have received an aminoglycoside tested negative for the m1555A&gt;G mutation, 100% of patients who tested positive for the m1555A&gt;G mutation have not received an aminoglycoside, either before or after the test result.Results3815 patients were tested for the m.1555A&gt;G mutation between 2008–2018 and included for audit, 12 patients (0.31%) tested positive for the genetic mutation100% of these patients had their positive result recorded as an aminoglycoside allergy on JAC5 of these 12 patients had been treated with aminoglycosides at GOSH (42%)1 of these patients received Amikacin before genetic testingConclusionThis audit will inform future decisions regarding Trust guidelines containing amikacin. The low incidence of this mutation will be useful in discussion on moving forward in providing testing for patients. It reassures users that the current reporting system is robust and results are reliably recorded. Of the 5 patients who had a positive genetic mutation and received an aminoglycoside it was not clear if this was an intentional, informed risk benefit decision. Further work is being done to ensure these patients have and are followed up.Referenceshttps://www.medicines.org.uk/emc/product/3784/smpcEstivill X, Govea N, Barceló A, Perelló E, Badenas C, Romero E, et al. Familial Progressive Sensorineural Deafness Is Mainly Due to the mtDNA A1555G Mutation and Is Enhanced by Treatment with Aminoglycosides. The American Journal of Human Genetics. 1998;62:27–35.Göpel W, Berkowski S, Preuss M, et al. Mitochondrial mutation m.1555A&gt;G as a risk factor for failed newborn hearing screening in a large cohort of preterm infants. BMC Pediatrics. 2014;14:210.Bitner-Glindzicz M, Pembrey M, Duncan A, Heron J, Ring S, Hall A, et al. Prevalence of Mitochondrial 1555A→G Mutation in European Children. New England Journal of Medicine. 2009;360:640–642.

Follow-up after failed newborn hearing screening: Parental and primary care provider awareness.

The current loss to follow-up rate after failed newborn hearing screening (NBHS) is 34.4%. Previous studies have found that lack of parental and primary care provider (PCP) awareness of NBHS results are significant contributors to loss to follow-up. The objective of this study was to identify factors associated with parental and PCP awareness of NBHS results. Retrospective cohort study. A survey asking about demographics and knowledge of NBHS testing and results was offered to parents in the waiting room of an urban pediatric primary care office. Included were biological parents ≥18years of age of children ≤10years of age born in Pennsylvania. Each child's chart was reviewed for PCP documentation of NBHS results. The odds of knowing NBHS results were evaluated using logistic regression. The survey was completed by 304 parents. 74.0% were aware of their child's NBHS results. Child age ≥1year old (OR: 0.49, 95%CI[0.29, 0.82], P=0.007) and Hispanic ethnicity (OR: 0.38, 95%CI[0.16, 0.89], P=0.03) were associated with decreased odds of a parent knowing NBHS results. In addition, fewer fathers knew the results of their child's NBHS compared with mothers (OR: 0.33, 95%CI[0.18, 0.62], P<0.001). However, parental awareness was not associated with birthing facility or insurance type. 222 charts were reviewed for NBHS documentation, revealing PCP awareness in 95.5% of cases and no associations with any of the factors examined. Factors associated with parents not knowing NBHS results included being the parent of an older child, Hispanic, or the father.

Readability of online patient education materials for parents after a failed newborn hearing screen

ObjectiveA cross-sectional study design was utilized to evaluate the readability of patient education materials on the newborn hearing screen from Google and major institutions. MethodsThe top 55 websites from the Google search “failed newborn hearing screen” and websites from major institutions (the U.S. News & World Report ranked top 10 children's hospitals, the top 5 pediatric otolaryngology fellowships as ranked by Doximity Residency Navigator, the Centers for Disease and Control, the American Academy of Pediatrics and the American Academy of Otolaryngology–Head and Neck Surgery) were compiled. Text from each website was edited to remove extraneous text. Readability grade was calculated using the Flesch-Kincaid Grade Level (FKGL), Flesch Reading Ease Score, Gunning-Fog Index (GFI), Simple Measure of Gobbledygook (SMOG), Coleman-Liau Index, and Automated Readability Index. Intra- and inter-observer reliability were assessed. Results26 websites from Google and 29 websites from major institutions were evaluated. From Google, provider-oriented websites (n = 2) were more difficult to read than patient-oriented websites (n = 24) with statistical significance for FKGL (p < 0.001), GFI (p < 0.013) and SMOG (p < 0.001). From the major institutions, more than half were at a reading level that exceeded the average American adult with an average FKGL 9.71 ± 2.69. Intra- and inter-observer reliability were both excellent with an intra-class correlation coefficient for each readability tool ≥0.950 (p < 0.001). ConclusionOnline patient education materials about the newborn hearing screen may be too difficult for the average reader. Revisions to these materials and redirection to more readable online resources may be necessary to benefit a more inclusive patient population.

An evidence based protocol for managing neonatal middle ear effusions in babies who fail newborn hearing screening

ObjectivesTo evaluate the prevalence of middle ear disease in infants referred for failed newborn hearing screening (NBHS) and to review patient outcomes after intervention in order to propose an evidence-based protocol for management of newborns with otitis media with effusion (OME) who fail NBHS. Methods85 infants with suspected middle ear pathology were retrospectively reviewed after referral for failed NBHS. All subjects underwent a diagnostic microscopic exam with myringotomy with or without placement of a ventilation tube in the presence of a middle ear effusion and had intra-operative auditory brainstem response (ABR) testing or testing at a later date. ResultsAt the initial office visit, a normal middle ear space bilaterally was documented in 5 babies (6%), 29/85 (34%) had an equivocal exam while 51/85 (60%) had at least a unilateral OME. Myringotomy with or without tube placement due to presence of an effusion was performed on 65/85 (76%) neonates. Normal hearing was established in 17/85 (20%) after intervention, avoiding the need for any further audiologic workup. Bilateral or unilateral sensorineural hearing loss (SNHL) or mixed hearing loss was noted in 54/85 (64%) and these children were referred for amplification. Initially observation with follow up outpatient visits was initiated in 27/85 (32%) however, only 3/27 (11%) resolved with watchful waiting and 24/27 (89%) ultimately required at least unilateral tube placement due to OME and 14/24 (59%) were found to have at least a unilateral mixed or SNHL. ConclusionsAn effective initial management plan for children with suspected middle ear pathology and failed NBHS is diagnostic operative microscopy with placement of a ventilation tube in the presence of a MEE along with either intra-operative ABR or close follow-up ABR. This allows for the identification and treatment of babies with a conductive component due to OME, accurate diagnosing of an underlying SNHL component and for prompt aural rehabilitation.

Results of a Targeted Screening Program for Congenital Cytomegalovirus Infection in Infants Who Fail Newborn Hearing Screening.

Congenital cytomegalovirus (CMV) infection is a major cause of sensorineural hearing loss. By law, newborns in Connecticut who fail newborn hearing screening are tested for infection with CMV. This targeted screening is controversial, because most children with congenital CMV infection are asymptomatic, and CMV-related hearing loss can have a delayed onset. Our hospital uses a saliva polymerase chain reaction (PCR) assay (confirmed by a urine PCR assay) to detect CMV. Here, we report the results of the first year of our screening program. We reviewed the medical records of newborns in the Yale New Haven Health System who failed the newborn hearing screening test between January 1 and December 31, 2016. Of 10964 newborns, 171 failed newborn hearing screening, and 3 of these newborns had positive saliva CMV PCR test results. Of these 3 newborns, 2 had positive results on the confirmatory test (for 1 of them the confirmatory test was not performed until the infant was 10 weeks old), and 1 had a negative result on the confirmatory test. Three additional newborns with congenital CMV infection were tested because of clinical indications (1 for ventriculomegaly on prenatal ultrasound and 2 for CMV infection of the mother). Results of audiology follow-up were available for 149 (87.1%) of the 171 newborns who failed newborn hearing screening; 127 (85.2%) had normal results. Our targeted screening program for congenital CMV infection had a low yield. Consideration should be given to other strategies for identifying children at risk of hearing loss as a result of congenital CMV infection.

Infant, Maternal, and Hospital Factors' Role in Loss to Follow-up After Failed Newborn Hearing Screening

ObjectiveCompletion of newborn hearing screening (NBHS) is recommended by 1 month old. Delays and loss to follow-up and documentation (LTF/LTD) after failed NBHS are common. Committees of experts have established hospital guidelines to reduce LTF/LTD. We aimed to identify maternal and infant factors associated with LTF/LTD and determine if adherence to hospital guidelines is associated with timely completion of follow-up screening. MethodsWe conducted a retrospective study of all infants born in Colorado hospitals who failed the newborn admission hearing screening from 2007 to 2012 and a cross-sectional survey of NBHS coordinators at Colorado birthing hospitals. Neonatal intensive care unit infants were excluded. Outcomes included documented completion of the follow-up NBHS and completion by 1 month. Data sources comprised the electronic birth record, infant hearing integrated data system, and NBHS coordinator survey. Data were analyzed by logistic regression. ResultsA total of 13,904 newborns did not pass the newborn admission hearing screening from 2007 to 2012, and 11,422 (82%) had documentation of a completed follow-up screening. A total of 10,558 (76%) completed follow-up screening by 1 month. All 53 NBHS coordinators completed the survey. Maternal age, education, smoking, and birth country; and payer, race, birth order, and population density were associated with completion of follow-up hearing screening. Maternal education, payer, population density, birth weight, and cleft lip were associated with completion by 1 month of age. Only birth in a facility that charges a rescreening fee was associated with completion of follow-up screening. ConclusionsLow-income, rural, and minority infants are at risk for LTF. Further studies are needed to determine if adherence to guidelines can overcome barriers to follow-up.

Mitochondrial mutation m.1555A>G as a risk factor for failed newborn hearing screening in a large cohort of preterm infants.

BackgroundThe mitochondrial m.1555A>G mutation is associated with a high rate of permanent hearing loss, if aminoglycosides are given. Preterm infants have an increased risk of permanent hearing loss and are frequently treated with aminoglycoside antibiotics.MethodsWe genotyped preterm infants with a birth weight below 1500 grams who were prospectively enrolled in a large cohort study for the m.1555A>G mutation. Treatment with aminoglycoside antibiotics in combination with mitochondrial m.1555A>G mutation was tested as a predictor for failed hearing screening at discharge in a multivariate logistic regression analysis.Results7056 infants were genotyped and analysed. Low birth weight was the most significant predictor of failed hearing screening (p = 7.3 × 10-10). 12 infants (0.2%) had the m.1555A>G-mutation. In a multivariable logistic regression analysis, the combination of aminoglycoside treatment with m.1555A>G-carrier status was associated with failed hearing screening (p = 0.0058). However, only 3 out of 10 preterm m.1555A>G-carriers who were exposed to aminoglycosides failed hearing screening. The m.1555A>G-mutation was detected in all mothers of m.1555A>G-positive children, but in none of 2993 maternal DNA-samples of m.1555A>G-negative infants.ConclusionAntenatal screening for the m.1555A>G mutation by maternal genotyping of pregnant women with preterm labour might be a reasonable approach to identify infants who are at increased risk for permanent hearing loss. Additional studies are needed to estimate the relevance of cofactors like aminoglycoside plasma levels and birth weight and the amount of preterm m.1555A>G-carriers with permanent hearing loss.

G155(P) A service evaluation of routes of identification of childhood hearing loss, post the new born screening programme

ObjectiveTo identify and evaluate the routes through which children with hearing loss are identified post the newborn hearing screening programme, in a chosen healthcare trust.MethodEstablish a database of a sample...

Seromukotympanon

Otitis media with effusion (OME) as the most common cause of hearing loss in childhood plays an important role in the follow-up after failed newborn hearing screening (NHS). The introduction of the NHS allows OME to be diagnosed and treated in the first months of life. In 715 children referred to the Department of Phoniatrics and Pedaudiology of the Ear, Nose, Throat University Hospital of Magdeburg during the period 2006-2010, the prevalence and therapy process of OME during follow-up could be determined. OME was diagnosed in 16 % of the patients. Spontaneous resolution was observed in 37 % of the cases, while 46 % of the children were treated surgically. A substantially increased risk for persistent OME was observed in children with craniofacial dysmorphia or cleft palate. Using NHS, OME can be diagnosed and treated early, thus, preventing potential problems in the linguistic, social and intellectual development of children.

Factors Influencing Follow-Up to Newborn Hearing Screening for Infants Who Are Hard of Hearing

To document the epidemiological characteristics of a group of children who are hard of hearing, identify individual predictor variables for timely follow-up after a failed newborn hearing screening, and identify barriers to follow-up encountered by families. The authors used an accelerated longitudinal design to investigate outcomes for children who are hard of hearing in a large, multicenter study. The present study involved a subgroup of 193 children with hearing loss who did not pass the newborn hearing screening. The authors used available records to capture ages of confirmation of hearing loss, hearing aid fitting, and entry into early intervention. Linear regression models were used to investigate relationships among individual predictor variables and age at each follow-up benchmark. Of several predictor variables, only higher levels of maternal education were significantly associated with earlier confirmation of hearing loss and fitting of hearing aids; severity of hearing loss was not. No variables were significantly associated with age of entry into early intervention. Each recommended benchmark was met by a majority of children, but only one third met all of the benchmarks within the recommended time frame. Results suggest that underserved communities need extra support in navigating steps that follow failed newborn hearing screening.

Detection of Cytomegalovirus DNA in Dried Blood Spots of Minnesota Infants Who Do Not Pass Newborn Hearing Screening

Up to 15% of infants with asymptomatic congenital cytomegalovirus (CMV) infection will experience some degree of sensorineural hearing loss. Many infants who fail newborn hearing screening (NHS) are likely to have congenital CMV infection, but may escape definitive virologic identification because diagnostic evaluation may not commence until several weeks or months of age, making differentiation between congenital and postnatal CMV infection difficult. Early diagnosis linking virologic identification of congenital CMV infection to infants failing NHS may improve diagnostic precision and enhance opportunities for therapeutic intervention. The goal of this study was to compare newborn dried blood spots from Minnesota infants who had failed NHS, and were designated for referral, with control infants who passed NHS, for the presence of CMV DNA by real-time PCR, using hybridization probes for the CMV gene UL54. Of 479 infants with a failed NHS (bilateral failure), 13 had CMV DNA present in the blood spot (2.7%). This compared with only 2/479 positive results from a control group of infants who passed the NHS (0.4%; P = 0.007, Fisher exact test). Comparisons of the glycoprotein B (gB) genotype as well as direct DNA sequencing of selected positives revealed that PCR positive samples represented unique clinical isolates. The mean viral load among the 15 positive samples was 1.6 x 10(3) genomes/microgram of total DNA. Newborn bloodspot CMV screening by real-time PCR may be a useful and rapid adjunct to functional NHS and may enable more rapid etiologic diagnosis of sensorineural hearing loss in newborns.

State Laws Improve Hearing Screening Follow-Up

State Laws Improve Hearing Screening Follow-Up